Background/Purpose: The Ehlers-Danlos Syndromes (EDS) are a heritable group of connective tissue disorders with predominant features including joint hypermobility, skin hyperextensibility and tissue fragility. Hypermobility Spectrum Disorders (HSD) include all phenotypes presenting with joint hypermobility plus one or more of its secondary manifestations but not satisfying the criteria for EDS (Castori, 2017). Common symptoms reported by patients with EDS and HSD include pain and fatigue, resulting in decreased function, including lack of exercise. It has been shown that patients with EDS and HSD overall improve with exercise interventions (Palmer, 2014).The purpose of this study was explore relationships between self-reported pain, fatigue, and function; with perceived benefits and barriers to exercise.

Methods: A retrospective chart review was completed on 38 consecutive patients with either EDS or HSD to determine if there were any relationships between the scores for pain (high scores indicate greater pain), fatigue (high scores indicate greater fatigue), and function (low scores indicate greater function), as per the Multi-Dimensional Health Assessment Questionnaire (MD- HAQ); and the total score (high scores indicate more positive perception of exercise), benefits score (high scores indicate more positive perception of exercise), and barriers score (high scores indicate greater perception of barriers to exercise) on the Exercise Benefits and Barriers Scale (EBBS). These instruments were administered to patients as part of routine care to assist with treatment planning and recommendations. Descriptive statistics and univariate analysis (Pearson correlation coefficients) were used to determine if relationships existed between self-reported pain, fatigue and function; and perceived benefits scores, barriers scores, and total EBBS scores reported by patients.

Results: The majority of the sample was female (96 %); and the average age was 33.5 years. According to the 2017 International Classification for EDS, 23 patients had EDS (hypermobility type n=20; classical type n=1; vascular type n=1; kyphoscoliotic type n=1) and15 patients had HSD. Overall, EBBS scores indicated high perceived value of exercise. There was no relationship between fatigue (r = -0.143; p = 0.39) nor pain (r = -0.192; p = 0.25) with total EBBS scores. However, the higher patients reported their level of function, the higher were their total EBBS scores (r = 0.392; p = 0.015). Similarly, higher function scores, correlated with higher total benefits scores (r = 0.383; p = 0.018); and patients who reported higher function reported fewer perceived barriers to exercise (r = -0.44; p = 0.006).

Conclusion: These results support previously reported importance of exercise in the management of patients with EDS and HSD; and further highlight the critical role of programs to educate patients, with EDS and HSD regarding the importance of exercise, who are experiencing difficulty with function.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/pain-fatigue-and-function-in-patients-with-ehlers-danlos-syndrome-and-hypermobility-spectrum-disorder-relationship-with-perceived-benefits-and-barriers-to-exercise/